1、Adult Congenital Heart Disease “Common” Complex Lesions,Jeffrey D. Solomon, MDNovember 14 & 19, 2008,Objectives,Describe the changing profile of ACHD Describe the challenges in the transfer of care from pediatric to adult practitioners Review the development of the heart Describe the anatomy, natura
2、l history, surgical repair, and common adult presentations of tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome,ACHD,A gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance Overa
3、ll incidence: 75 of every 1000 live births,ACHD,A gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance Overall incidence: 75 of every 1000 live births Incidence of moderate and severe CHD: 8 of every 1000 live births,ACHD
4、,A gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance Overall incidence: 75 of every 1000 live births Incidence of moderate and severe CHD: 8 of every 1000 live births Over 90% of children survive to adulthood 20,000 op
5、erations for CHD in the US each year Over 1 million adults in the US with CHD,Transfer/Transition of Care,Challenges Complex CHD is seldom cured Patients have a poor understanding Timing of transition Lack of exposure among adult cardiologists,Transfer/Transition of Care,32nd Bethesda Conference (20
6、00) Regular evaluation at an ACHD center Health care passport Structured transfer/transition plan Co-management with cardiologists having undergone advanced training,Tetralogy of Fallot (TOF),Named by Etienne-Louis Arthur Fallot in 1888 Approximately 10% of all complex CHD Single developmental error
7、 of the terminal portion of the spiral truncoconal septum Four distinct components: subpulmonic stenosis, VSD, overriding aorta, and RV hypertrophy Often accompanied by other anomalies,Natural History,Depends on severity of RVOT obstruction Cyanosis and exertional dyspnea “Tet spell” profound cyanot
8、ic episode secondary to augmented right-to-left shunt Without surgical correction, patients do poorly,Surgical Repair,Longest surgical history/most studied outcomes Palliative surgical shunts,Surgical Repair,Longest surgical history/most studied outcomes Palliative surgical shunts Classic/complete r
9、epair Infundibular muscle resection, VSD patch, & RVOT repair Typically preformed between 4-6 months of age Surgical risk: 5% Survival rates: 85% at 30+ years,Adult Presentation,Repaired RVOT obstruction Pulmonary or tricuspid regurgitation,Adult Presentation,Repaired RVOT obstruction Pulmonary or t
10、ricuspid regurgitation LV/RV dysfunction Atrial/ventricular arrhythmias Unrepaired Significant morbidity Consider later repair,Transposition of the Great Arteries (TGA),Complete TGA or D-TGA Embryological inversion of the great arteries Ventriculoarterial discordance,Transposition of the Great Arter
11、ies (TGA),Complete TGA or D-TGA Embryological inversion of the great arteries Ventriculoarterial discordance Congenitally corrected TGA or L-TGA Embryological inversion of the ventricles Atrioventricular & ventriculoarterial discordance (double discordance),Surgical Repair,Balloon atrial septostomy
12、Developed by Rashkind (1965) Enlarges the atrial communication Atrial switch Performed first by Senning (1958) and later modified by Mustard (1964) Atrial baffle is created to direct venous return to the contralateral ventricle,Adult Presentation,RV dysfunction Tricuspid insufficiency Bradyarrhythmi
13、as Atrial tachycardias Obstructed/leaky atrial baffle,Surgical Repair,Arterial switch Performed first by Jatene (1976) Great arteries transected and reattached to appropriate AV valve Coronary ostia also transplanted Surgical treatment of choice Excellent outcomes so far,Adult Presentation,Coronary
14、perfusion issues Supravalvar aortic and pulmonic stenosis Aortic root dilatation and valvular insufficiency Branch pulmonary stenosis,Single Ventricle Physiology,Hypoplastic left-heart syndrome Hypoplastic right-heart syndrome (pulmonary atresia with intact ventricular septum) Tricuspid valve atresi
15、a Mitral valve atresia Double-inlet ventricle Atrioventricular canal defects Double outlet right ventricle,Surgical Repair,Staged rerouting of the systemic venous return Step One Complete mixing of systemic and pulmonary venous return Unobstructed outflow tract to the systemic arterial circulation R
16、eliable source of pulmonary blood flow,Adult Presentation,RV dysfunction Cyanosis Protein losing enteropathy,Key Points,The majority of children born with CHD survive into adulthood Infants born with complex CHD are seldom cured; residual problems are common and predictable There are many challenges
17、 in the transition of care from pediatric to adult practitioners Adults with moderate and complex CHD should undergo regular evaluation at a regional ACHD center,Selected References,Bashore TM. Adult congenital heart disease: right ventricular outflow tract lesions. Circulation 2007 115: 1933-1947.
18、Gewillig M. The Fontan circulation. Heart 2005 91: 839-846. Hoffman JIE, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002 39: 1890-1900. Sommer RJ, Hijazi ZM, Rhodes JF. Pathophysiology of congenital heart disease in the adult: part III: complex congenital heart disease. C
19、irculation 2008 117: 1340-1350. Warnes CA. Transposition of the great arteries. Circulation 2006 114: 2699-2709. Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman JIE, Somerville J, William RG, Webb GD. Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol 2001 37: 1170-1175.,
